Dietary and circulating polyunsaturated fatty acids in Cystic Fibrosis : are they related to clinical outcomes?

Objective: To assess the relationship between dietary intakes, plasma phospholipid (PL) fatty acid profile and clin. parameters in children with cystic fibrosis (CF) in comparison to healthy controls. Patients and methods: A cross-sectional survey including 37 patients with CF (ages 8.0 +- 2.9 yrs) and a ref. group of 68 healthy children (ages 8.0 +- 0.7 yrs) was carried out by means of a food-frequency questionnaire. At enrolment, all subjects underwent blood sampling for plasma PL fatty acids (FA). In CF patients, pulmonary function tests (forced expiratory vol. in 1 s and forced vital capacity), anthropometric measurements and the Shwachman score were also detd. Results: In CF patients, mean z score for wt. and height (-0.35 +- 1.16 and -0.28 +- 0.99) were lower than controls (0.83 +- 1.73 and 0.55 +- 1.11, resp.). Patients with CF showed higher energy intakes (110 +- 43 kcal/d) compared with controls (75 +- 22 kcal/d; P < 0.0001), with higher intake of total (satd. and monounsatd.) fats and lower intake of polyunsatd. FA (3.9 +- 1.0% of total macronutrient intake vs 4.3 +- 1.2%, P= 0.05). In CF patients, plasma and PL levels of linoleic and docosahexaenoic acids were lower, whereas those of arachidonic acid were similar compared with controls. The Shwachman score showed significant pos. assocns. with plasma PL levels of arachidonic acid and total n-6 long-chain FA (r= 0.32, P= 0.05, and r= 0.35, P= 0.03, resp.). Conclusions: The data give suggestions that fat intake and CF-assocd. biomechanisms are bound in a vicious circle, concurring to create the clin. and biochem. picture of CF. The quantity and quality of fat supplementation in CF need careful attention to balance the fat supply with polyunsatd. FA.